Brief clinical report - New dysostosis showing multilevel absence of vertebral pedicles: Unique developmental anomaly of vertebral arches?

We report on an apparently normal child who shows hypopaplasia of the verte bral pedicles and posterior arches of several cervical, thoracic, and lumba r vertebrae with normally fused spinous apophyses, hypoplastic sacrum, lumb ar epidural lipomatosis, synostoses of some cervical vertebral disks, and s acral spina bifida, The most likely mechanism is an abnormal differentiatio n of the spinal processes, due most probably to an absence of differentiati on in cartilage of the dense mesenchyme forming their most anterior part. B ecause the anomalies affect multiple levels, we highly suspect a genetic ba sis to this unusual dysostosis affecting the development of the posterior s clerotomes. (C) 2000 Wiley-Liss, Inc.